骨髓单个核细胞Coombs试验阳性免疫相关性血细胞减少症72例临床分析

目的总结骨髓单个核细胞Coombs(BMMNC-Coombs)试验阳性免疫相关性血细胞减少症(IRP)患者的临床特征。方法回顾性分析天津医科大学总医院血液科1995年1月至2004年3月诊治的72例骨髓BMMNC-Coombs试验阳性的IRP患者的发病诱因、临床表现、血象及骨髓象、自身抗体类型及免疫治疗效果。结果62.5%(45/72)IRP患者发病前有感染、过敏或妊娠等诱因。72.2%(52/72)患者全血细胞减少;91.7%(66/72)患者有网织红细胞不低的大细胞或正细胞贫血;75.0%(54/72)患者有白细胞减少;18.1%(13/72)患者合并发热;95.8%(69/72)患者可见血小板轻、中、重度减少。以皮肤黏膜出血为主。髂骨骨髓增生活跃和明显活跃68.1%(49/72)],红系比例增高51.4%(37/72)]或正常19.4%(14/72)],骨髓淋巴细胞比例与疗效呈负相关;大部分患者65.9%(27/41)]胸骨骨髓为增生活跃或明显活跃。BMMNC-Coombs试验皆阳性,检出不同的自身抗体类型和组合方式,56.9%(41/72)患者有溶血迹象,但不符合任何种类的溶血性疾病的诊断标准;42.9%(18/42)患者补体C3降低,19.0%(8/42)患者同时或单独存在补体C4降低。给予免疫抑制和促造血治疗后,3年有效率为87.5%(28/32)。结论IRP是一类获得性自身抗体介导的骨髓细胞破坏或抑制性疾病,BMMNC-Coombs试验阳性IRP患者主要表现为网织红细胞和(或)中性粒细胞比例不低的2系或3系血细胞减少,对免疫抑制和促造血治疗反应好。

Clinical analysis of 72 cases of immunorelated pancytopenia with positive BMMNC-Coombs tests

Objective To study the clinical and laboratory features of the patients with immunorelated pancytopenia(IRP).Methods The risk factors,manifestations,blood cell counts,bone marrow phenotypes,autoantibodies and immunosuppressive therapy response of 72 patients with IRP were analyzed.Then they were followed up for(6 ~36)months,to see their long-term outcome and the prognostic factors.Results The infection,anaphylaxis and pregnancy were highly suspected to be the risk factors of IRP.Most of these patients were with pancytopenia72.2%(52/72);91.7%(66/72)of them were anemic with large or normal MCV;75.0%(54/72)of them were with leukopenia;18.1%(13/72)of them had fever.Thrombocytopenia was common,but serious bleeding rare.68.1% of these cases were with normal or decreased bone marrow cellularities and increased normoblasts.They were all found to have positive results of bone marrow mononuclear cell Coombs test,negative results of routine hemolysis tests and no evidence of malignant clonal hematopoiesis.42.9%(18/42)of these patients had C3 decreased,and 19.0%(8/42)C4 decreased.Immunosuppressive therapy was administered to 72 IRP patients.The response rate at 36 months was 87.5%(28/32).Conclusion IRP is a blood disease induced by acquired autoantibodies mediated bone marrow inhibition or destruction.It manifests pancytopenia with relative higher percentages of reticulocytes and neutrophils,and responds well to immunosuppressive therapy.